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Subclinical systolic dysfunction in left ventricular non-compaction cardiomyopathy unmasked by contrast echocardiography
International Journal of Cardiology 172 (2014) e393–e395
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Subclinical systolic dysfunction in left ventricular non-compaction cardiomyopathy unmasked by contrast echocardiography José Alberto de Agustín ⁎, Jose Juan Gomez de Diego, Jose Luis Rodrigo, Pedro Marcos-Alberca, Carlos Almeria, Ivan Javier Nuñez-Gil, Maria Luaces, Miguel Angel Garcia-Fernandez, Carlos Macaya, Leopoldo Perez de Isla Cardiovascular Institute, Hospital Universitario San Carlos, Madrid, Spain
a r t i c l e
i n f o
Article history: Received 21 November 2013 Accepted 30 December 2013 Available online 11 January 2014 Keywords: Subclinical systolic dysfunction Left ventricular non-compaction Contrast echocardiography
An asymptomatic 28-year-old man, first-degree relative of a patient with left ventricular non-compaction (LVNC), underwent an echocardiographic screening. Two-dimensional transthoracic echocardiography demonstrated normal left ventricular (LV) dimensions (enddiastolic diameter of 49 mm) with apparently normal systolic function. Characteristic, prominent numerous trabeculations in the LV apex and inferior, posterior, and lateral wall were noticed (Fig. 1 and Movie I). The color Doppler imaging demonstrated communication between intertrabecular spaces and the LV cavity. Contrast echocardiography with intravenous administration of a contrast agent (Sonovue®) was performed, improving endocardial border definition and revealing the presence of mild–moderate global LV hypokinesis that went unnoticed in the 2D image without contrast (Fig. 2 and Movie II). The LV ejection fraction calculated using contrast echocardiography by the Simpson's method was 40%. The ratio of the non-compacted to compacted zone during the end-systolic phase of the cardiac cycle was 4.2. Cardiac magnetic resonance was performed confirming the echocardiographic findings and the presence of moderate global LV systolic dysfunction (Fig. 3 and Movie III). The diagnosis of LVNC was established and the patient was started on enalapril and carvedilol. Left ventricular non-compaction is a disorder of endomyocardial morphogenesis that results in excessive and prominent LV trabeculations associated with systolic dysfunction in advanced disease. The classic clinical presentation includes heart failure, malignant ventricular arrhythmias, thromboembolic events and sudden cardiac death [1].
⁎ Corresponding author. Tel.: +34 913303394; fax: +34 913303290. E-mail address: [email protected] (J.A. de Agustín). 0167-5273/$ – see front matter © 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.266
Prognosis is worse in patients with LV systolic dysfunction. In this group of patients cardiac resynchronization therapy or placement of an implantable cardioverter-defibrillator may improve long-term survival [2]. Prevention of embolic complications is an additional important issue that should be taken under consideration, and several authors have recommended long-term prophylactic anticoagulation in the presence of LV systolic dysfunction [3]. Therefore early identification of LV dysfunction is crucial for adequate treatment and prevention of possible complications. Echocardiography is the most widely used diagnostic technique to establish a diagnosis and determine a treatment plan. Although LVNC was initially described in patients with severe LV systolic dysfunction [2], normal or near-normal LV systolic function has been increasingly reported [4], particularly in asymptomatic patients detected by familial screening [5]. As demonstrated in our case, contrast echocardiography improves endocardial border definition, providing a more accurate assessment of LV volumes and function, and enabling detection of subclinical systolic dysfunction. Therefore, contrast echocardiography should be routinely performed in patients with LVNC, particularly in those with apparently normal LV systolic function. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2013.12.266. References [1] Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;86:666–71. [2] Oechslin EN, Attenhofer Jost CH, Rojas JR, Kauffman P, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000;36:493–500. [3] Borges AC, Kivelitz D, Baumann G. Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 2003;89:21. [4] Fazio G, Sutera L, Corrado G, Novo S. The chronic heart failure is not so frequent in non-compaction. Eur Heart J 2007;28:1269. [5] Habib G, Charron P, Eicher JC, et al. Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail 2011;13:177–85.
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J.A. de Agustín et al. / International Journal of Cardiology 172 (2014) e393–e395
Fig. 1. Transthoracic echocardiography in apical four chamber (A) and three chamber (B) views. Numerous prominent trabeculations in combination with deep intertrabecular spaces in the apex and inferior, posterior, and lateral ventricular wall were observed. Color Doppler echocardiography demonstrated communication between intertrabecular spaces and the left ventricular cavity (C and D). LV indicates left ventricle; and RV right ventricle.
Fig. 2. Contrast echocardiography improved endocardial border definition and unmasked the presence of mild–moderate global left ventricular hypokinesis, with a calculated ejection fraction of 40% by the Simpson's method (A and B). EDV indicates end-diastolic volume; ESV end-systolic volume; and LVEF left ventricular ejection fraction.
J.A. de Agustín et al. / International Journal of Cardiology 172 (2014) e393–e395
e395
Fig. 3. Cardiac magnetic resonance in four chamber (A) and mid-ventricular short axis (B) views, confirming diagnosis of left ventricular non-compaction and the presence of moderate global LV systolic dysfunction. LV indicates left ventricle; and RV right ventricle.
Contents lists available at ScienceDirect
International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard
Letter to the Editor
Subclinical systolic dysfunction in left ventricular non-compaction cardiomyopathy unmasked by contrast echocardiography José Alberto de Agustín ⁎, Jose Juan Gomez de Diego, Jose Luis Rodrigo, Pedro Marcos-Alberca, Carlos Almeria, Ivan Javier Nuñez-Gil, Maria Luaces, Miguel Angel Garcia-Fernandez, Carlos Macaya, Leopoldo Perez de Isla Cardiovascular Institute, Hospital Universitario San Carlos, Madrid, Spain
a r t i c l e
i n f o
Article history: Received 21 November 2013 Accepted 30 December 2013 Available online 11 January 2014 Keywords: Subclinical systolic dysfunction Left ventricular non-compaction Contrast echocardiography
An asymptomatic 28-year-old man, first-degree relative of a patient with left ventricular non-compaction (LVNC), underwent an echocardiographic screening. Two-dimensional transthoracic echocardiography demonstrated normal left ventricular (LV) dimensions (enddiastolic diameter of 49 mm) with apparently normal systolic function. Characteristic, prominent numerous trabeculations in the LV apex and inferior, posterior, and lateral wall were noticed (Fig. 1 and Movie I). The color Doppler imaging demonstrated communication between intertrabecular spaces and the LV cavity. Contrast echocardiography with intravenous administration of a contrast agent (Sonovue®) was performed, improving endocardial border definition and revealing the presence of mild–moderate global LV hypokinesis that went unnoticed in the 2D image without contrast (Fig. 2 and Movie II). The LV ejection fraction calculated using contrast echocardiography by the Simpson's method was 40%. The ratio of the non-compacted to compacted zone during the end-systolic phase of the cardiac cycle was 4.2. Cardiac magnetic resonance was performed confirming the echocardiographic findings and the presence of moderate global LV systolic dysfunction (Fig. 3 and Movie III). The diagnosis of LVNC was established and the patient was started on enalapril and carvedilol. Left ventricular non-compaction is a disorder of endomyocardial morphogenesis that results in excessive and prominent LV trabeculations associated with systolic dysfunction in advanced disease. The classic clinical presentation includes heart failure, malignant ventricular arrhythmias, thromboembolic events and sudden cardiac death [1].
⁎ Corresponding author. Tel.: +34 913303394; fax: +34 913303290. E-mail address: [email protected] (J.A. de Agustín). 0167-5273/$ – see front matter © 2014 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.12.266
Prognosis is worse in patients with LV systolic dysfunction. In this group of patients cardiac resynchronization therapy or placement of an implantable cardioverter-defibrillator may improve long-term survival [2]. Prevention of embolic complications is an additional important issue that should be taken under consideration, and several authors have recommended long-term prophylactic anticoagulation in the presence of LV systolic dysfunction [3]. Therefore early identification of LV dysfunction is crucial for adequate treatment and prevention of possible complications. Echocardiography is the most widely used diagnostic technique to establish a diagnosis and determine a treatment plan. Although LVNC was initially described in patients with severe LV systolic dysfunction [2], normal or near-normal LV systolic function has been increasingly reported [4], particularly in asymptomatic patients detected by familial screening [5]. As demonstrated in our case, contrast echocardiography improves endocardial border definition, providing a more accurate assessment of LV volumes and function, and enabling detection of subclinical systolic dysfunction. Therefore, contrast echocardiography should be routinely performed in patients with LVNC, particularly in those with apparently normal LV systolic function. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2013.12.266. References [1] Jenni R, Oechslin E, Schneider J, Attenhofer Jost C, Kaufmann PA. Echocardiographic and pathoanatomical characteristics of isolated left ventricular noncompaction: a step towards classification as a distinct cardiomyopathy. Heart 2001;86:666–71. [2] Oechslin EN, Attenhofer Jost CH, Rojas JR, Kauffman P, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol 2000;36:493–500. [3] Borges AC, Kivelitz D, Baumann G. Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion. Heart 2003;89:21. [4] Fazio G, Sutera L, Corrado G, Novo S. The chronic heart failure is not so frequent in non-compaction. Eur Heart J 2007;28:1269. [5] Habib G, Charron P, Eicher JC, et al. Isolated left ventricular non-compaction in adults: clinical and echocardiographic features in 105 patients. Results from a French registry. Eur J Heart Fail 2011;13:177–85.
e394
J.A. de Agustín et al. / International Journal of Cardiology 172 (2014) e393–e395
Fig. 1. Transthoracic echocardiography in apical four chamber (A) and three chamber (B) views. Numerous prominent trabeculations in combination with deep intertrabecular spaces in the apex and inferior, posterior, and lateral ventricular wall were observed. Color Doppler echocardiography demonstrated communication between intertrabecular spaces and the left ventricular cavity (C and D). LV indicates left ventricle; and RV right ventricle.
Fig. 2. Contrast echocardiography improved endocardial border definition and unmasked the presence of mild–moderate global left ventricular hypokinesis, with a calculated ejection fraction of 40% by the Simpson's method (A and B). EDV indicates end-diastolic volume; ESV end-systolic volume; and LVEF left ventricular ejection fraction.
J.A. de Agustín et al. / International Journal of Cardiology 172 (2014) e393–e395
e395
Fig. 3. Cardiac magnetic resonance in four chamber (A) and mid-ventricular short axis (B) views, confirming diagnosis of left ventricular non-compaction and the presence of moderate global LV systolic dysfunction. LV indicates left ventricle; and RV right ventricle.